Cognition, attention, and behavior in Prader-Willi syndrome

We studied the academic, cognitive, and behavior profile of 18 patients wit h Prader-Willi syndrome. All had severe learning disabilities in arithmetic and writing, and the majority were also dyslexic. Their average Full-Scale IQ was 73.7 +/- 8.9, which was 1 SD below normal range, whereas their perf ormance on executive, memory, and visuospatial tasks ranged from 2.1 to 7.0 SD below the expected means. Behavioral problems were measured using the C hild Behavior Checklist, on which the majority scored in the pathologic ran ge for social and attention problems, delinquent and aggressive behavior, s omatic complaints, and thought problems. Genotypes of the children did not predict cognitive or behavioral profile, nor could behavior be associated w ith parameters of weight. or IQ. In summary, we found that patients with Pr ader-Willi syndrome have profound learning disabilities and cognitive defic its, greater than expected for their IQ. Behavioral problems, including att ention-deficit hyperactivity disorder (ADHD), are also prevalent and impede the overall management of this group of patients. The genotypes were not h elpful in predicting cognitive or behavioral patterns.